Specific Motor Signs Research Articles

Motor signs and incident dementia with Lewy bodies in older adults with mild cognitive impairment.

Motor signs may herald incident dementia and allow the earlier detection of high-risk individuals and the timely implementation of preventive interventions. The current study was performed to investigate the prognostic properties of motor signs with respect to incident dementia with Lewy bodies (DLB) in older adults with mild cognitive impairment (MCI). Emphasis was placed on sex differences. The specificity of these associations was explored. We analyzed data from the National Alzheimer's Coordinating Center Uniform Data Set. Participants 55 + years old with a diagnosis of MCI were included in the analysis. Those with Parkinson's disease (PD) or other parkinsonian disorders at baseline and those with PD dementia at follow-up were excluded. UPDRS III was used to assess the presence or absence of motor signs in nine domains: hypophonia; masked facies; resting tremor; action/postural tremor; rigidity; bradykinesia; impaired chair rise; impaired posture/gait; postural instability. Αdjusted Cox proportional hazards models featuring sex by motor sign interactions were estimated. Throughout the average follow-up of 3.7 ± 3.1 years, among 4623 individuals with MCI, 2211 progressed to dementia (66 of whom converted to DLB). Masked facies [HR = 4.21 (1.74-10.18)], resting tremor [HR = 4.71 (1.44-15.40)], and bradykinesia [HR = 3.43 (1.82-6.45)] exclusively increased the risk of DLB. The HR of DLB was approximately 15 times greater in women compared to men with masked facies. Impaired posture-gait (approximately 10 times) and resting tremor (approximately 8.5 times) exhibited a similar trend (prominent risk-conferring properties in women compared to men) but failed to achieve statistical significance. Rigidity and hypophonia elevated the risk of other dementia entities, as well. The remaining motor features were not related to incident dementia of any type. Specific motor signs may herald DLB among individuals with MCI. Different associations may exist between masked facies, impaired posture-gait, resting tremor, and incident DLB in men versus women.

Chronic adaptive deep brain stimulation versus conventional stimulation in Parkinson's disease: a blinded randomized feasibility trial.

Deep brain stimulation (DBS) is a widely used therapy for Parkinson's disease (PD) but lacks dynamic responsiveness to changing clinical and neural states. Feedback control might improve therapeutic effectiveness, but the optimal control strategy and additional benefits of 'adaptive' neurostimulation are unclear. Here we present the results of a blinded randomized cross-over pilot trial aimed at determining the neural correlates of specific motor signs in individuals with PD and the feasibility of using these signals to drive adaptive DBS. Four male patients with PD were recruited from a population undergoing DBS implantation for motor fluctuations, with each patient receiving adaptive DBS and continuous DBS. We identified stimulation-entrained gamma oscillations in the subthalamic nucleus or motor cortex as optimal markers of high versus low dopaminergic states and their associated residual motor signs in all four patients. We then demonstrated improved motor symptoms and quality of life with adaptive compared to clinically optimized standard stimulation. The results of this pilot trial highlight the promise of personalized adaptive neurostimulation in PD based on data-driven selection of neural signals. Furthermore, these findings provide the foundation for further larger clinical trials to evaluate the efficacy of personalized adaptive neurostimulation in PD and other neurological disorders. ClinicalTrials.gov registration: NCT03582891 .

Association between Motor Signs and Cognitive Performance in Cognitively Unimpaired Older Adults: A Cross-Sectional Study Using the NACC Database.

Aiming to examine whether specific motor signs are associated with worse performance in specific cognitive domains among cognitively unimpaired (CU) individuals, we performed a cross-sectional analysis of data from the baseline evaluations of older, CU participants from the National Alzheimer’s Coordinating Center (NACC) Uniform Data Set. In total, 8149 CU (≥60 years) participants were included. Of these, 905 individuals scored ≥ 2 on at least one of the motor domains of the Unified Parkinson’s Disease Rating Scale part III (UPDRSIII). Cognitively impaired individuals, participants with psychiatric disorders and/or under treatment with antipsychotic, anxiolytic, sedative or hypnotic agents were excluded. Nine motor signs were examined: hypophonia, masked facies, resting tremor, action/postural tremor, rigidity, bradykinesia, impaired chair rise, impaired posture/gait and postural instability. Their association with performance on episodic memory, semantic memory, language, attention, processing speed or executive function was assessed using crude and adjusted linear regression models. Individuals with impaired chair rise had worse episodic memory, semantic memory, processing speed and executive function, while those with bradykinesia had worse language, processing speed and executive function. Sensitivity analyses, by excluding participants with cerebrovascular disease or PD, or other Parkinsonism, produced similar results with the exception of the relationship between bradykinesia and language performance.

Perspective: Phase Amplitude Coupling-Based Phase-Dependent Neuromodulation in Parkinson's Disease.

Deep brain stimulation (DBS) is an effective surgical therapy for Parkinson’s disease (PD). However, limitations of the DBS systems have led to great interest in adaptive neuromodulation systems that can dynamically adjust stimulation parameters to meet concurrent therapeutic demand. Constant high-frequency motor cortex stimulation has not been remarkably efficacious, which has led to greater focus on modulation of subcortical targets. Understanding of the importance of timing in both cortical and subcortical stimulation has generated an interest in developing more refined, parsimonious stimulation techniques based on critical oscillatory activities of the brain. Concurrently, much effort has been put into identifying biomarkers of both parkinsonian and physiological patterns of neuronal activities to drive next generation of adaptive brain stimulation systems. One such biomarker is beta-gamma phase amplitude coupling (PAC) that is detected in the motor cortex. PAC is strongly correlated with parkinsonian specific motor signs and symptoms and respond to therapies in a dose-dependent manner. PAC may represent the overall state of the parkinsonian motor network and have less instantaneously dynamic fluctuation during movement. These findings raise the possibility of novel neuromodulation paradigms that are potentially less invasiveness than DBS. Successful application of PAC in neuromodulation may necessitate phase-dependent stimulation technique, which aims to deliver precisely timed stimulation pulses to a specific phase to predictably modulate to selectively modulate pathological network activities and behavior in real time. Overcoming current technical challenges can lead to deeper understanding of the parkinsonian pathophysiology and development of novel neuromodulatory therapies with potentially less side-effects and higher therapeutic efficacy.

Effect of levodopa on electroencephalographic biomarkers of the parkinsonian state.

The objective of this study was to evaluate proposed electroencephalographic (EEG) biomarkers of Parkinson's disease (PD) and test their correlation with motor impairment in a new, well-characterized cohort of PD patients and controls. Sixty-four-channel EEG was recorded from 14 patients with rigid-akinetic PD with minimal tremor and from 14 age-matched healthy controls at rest and during voluntary movement. Patients were tested off and on medication during a single session. Recordings were analyzed for phase-amplitude coupling over sensorimotor cortex and for pairwise coherence from all electrode pairs in the recording montage (distributed coherence). Phase-amplitude coupling and distributed coherence were found to be elevated Off compared with On levodopa, and their reduction was correlated with motor improvement. In the Off medication state, phase-amplitude coupling was greater in sensorimotor contacts contralateral to the most affected body part and reduced by voluntary movement. We conclude that phase-amplitude coupling and distributed coherence are cortical biomarkers of the parkinsonian state that are detectable noninvasively and may be useful as objective aids for management of dopaminergic therapy. Several analytic methods may be used for noninvasive measurement of abnormal brain synchronization in PD. Calculation of phase-amplitude coupling requires only a single electrode over motor cortex. NEW & NOTEWORTHY Several EEG biomarkers of the parkinsonian state have been proposed that are related to abnormal cortical synchronization. We report several new findings in this study: correlations of EEG markers of synchronization with specific motor signs of Parkinson's disease (PD), and demonstration that one of the EEG markers, phase-amplitude coupling, is more elevated over the more clinically affected brain hemisphere. These findings underscore the potential utility of scalp EEG for objective, noninvasive monitoring of medication state in PD.

Individual parkinsonian motor signs and striatal dopamine transporter deficiency: a study with [I-123]FP-CIT SPECT

IntroductionTotal parkinsonian motor symptom severity correlates with presynaptic striatal dopamine function in patients with Parkinson’s disease. There is a lack of studies that have investigated the associations between parkinsonian motor signs and striatal dopaminergic deficiency in patients with parkinsonism of an unknown origin. Identification of specific motor signs associated with the highest likelihood of striatal dopamine deficiency could aid the differential diagnostics of parkinsonian and tremor syndromes.MethodsIn this cross-sectional clinical and imaging study, detailed motor examinations were performed for 221 patients with parkinsonism or tremor of an unknown origin immediately before dopamine transporter (DAT) [I-123]FP-CIT SPECT imaging. Region-of-interest and voxel-based methods were used to investigate striatal DAT deficiency in relation to individual motor signs.ResultsUpper extremity rigidity and facial expression were the only motor signs that differentiated patients with normal and abnormal striatal DAT function. The presence of any upper extremity rigidity showed the highest likelihood of DAT deficiency (OR 4.79, 95% CI 1.56–14.75, P = 0.006) followed by reduced facial expression (OR 2.14, 95% CI 1.14–4.00, P = 0.018). In patients with DAT deficits, reduced facial expression was associated with DAT deficiency specifically in the caudate nucleus, and increased upper extremity rigidity was associated with DAT loss in the dorsal putamen (FWE-corrected P < 0.05).ConclusionsIncreased upper extremity muscle tone and hypomimia are independently associated with a higher likelihood of striatal hypodopaminergic imaging finding. This information can be used as a factor when the clinical need of auxiliary investigations, such as DAT SPECT, is considered for patients with parkinsonism.

Grey matter volume loss is associated with specific clinical motor signs in Huntington's disease

BackgroundMotor disturbances are clinical hallmarks of Huntington's disease (HD) and involve chorea, dystonia, hypokinesia and visuomotor dysfunction. Investigating the association between specific motor signs and different regional volumes is important to understand the heterogeneity of HD. ObjectiveTo investigate the motor phenotype of HD and associations with subcortical and cortical grey matter volume loss. MethodsStructural T1-weighted MRI scans of 79 HD patients and 30 healthy controls were used to calculate volumes of seven subcortical structures including the nucleus accumbens, hippocampus, thalamus, caudate nucleus, putamen, pallidum and amygdala. Multiple linear regression analyses, corrected for age, gender, CAG, MRI scan protocol and normalized brain volume, were performed to assess the relationship between subcortical volumes and different motor subdomains (i.e. eye movements, chorea, dystonia, hypokinesia/rigidity and gait/balance). Voxel-based morphometry analysis was used to investigate the relationship between cortical volume changes and motor signs. ResultsSubcortical volume loss of the accumbens nucleus, caudate nucleus, putamen, and pallidum were associated with higher chorea scores. No other subcortical region was significantly associated with motor symptoms after correction for multiple comparisons. Voxel-based cortical grey matter volume reductions in occipital regions were related with an increase in eye movement scores. ConclusionIn HD, chorea is mainly associated with subcortical volume loss, while eye movements are more related to cortical volume loss. Both subcortical and cortical degeneration has an impact on motor impairment in HD. This implies that there is a widespread contribution of different brain regions resulting in the clinical motor presentation seen in HD patients.

Basketball feint and non-verbal communication: empirical framework

Raiola, G. (2015). Basketball feint and non-verbal communication: empirical framework. J. Hum. Sport Exerc., 9(Proc1), pp.S360-S368. Bodily communication has its own epistemological framework in which the message follows a process of encoding, transmission and decoding. It establishes contacts and relationships that are developed first by data processing and after by message content evaluating. It has, within it, an ethnic-social substrate that changes in different cultures and contexts. It is a dynamic flow consisting of five basic elements: context, sender, recipient, channel, code and follows a logical and analogical process. The arguments of bodily communication affect the feint, which means manifest intention of achieving a goal through the initial implementation of a plan with specific motor signs, postures, attitudes, which are later implemented in totally different pursuance It refers to the “tactics” decoding of bodily communication. In this way, non verbal communication have to be included both in experimental and human sciences, so it can investigated by observation and data collected and get together the quantitative and qualitative aspects. The game situations in basketball are so many variables, determined from interacting behaviors engines all players, between teammates and against the opponents. The behaviors of the feints are of great importance both that quality execution technique that every player has and to choice the tactical option. In this study, it is request to recognize the main aspects in basketball, such as in volleyball in past study and to give an argumentative and deductive classification. Method is the observation by descriptive research of three experts: coach of team, experts of body communication and performance analyst. Results show some interested aspects. The body feint is always a deliberate attempt to deceive the opponent to gain an advantage in the context of the game, moreover, it is the basis of the game of basketball, along one players against one, on which is built the complex process of acquisition of tactical advantages over opponents. There are some prerequisites to be able to realize the motor actions of fake, definitely a good knowledge of the rules of the game, knowing how to hesitate (take time before changing direction) or anticipate a move (make a cut changing speed) and especially the ability to read situations game tactics. In conclusion, for young athletes, the education of these aspects becomes essential to ensure the development of the imagination and anticipation motor, which imply the ability of invention and adaptation to different situations of the game. The more a player forces the opponent to react to his feint (shooting, passing or departure dribble), instead of acting, the more likely will have to gain the benefits (time and space) in order to carry out his plan motor effectively (to make an easy score). Although, it is to highlight that, in the modern basketball defensive tactics (zone press, help and recovery, doublings, defensive switch, etc.) start to work out the following game situations: the defender must not have more behavior liabilities compared to the attacker, but in turn must perform the behavior of pretending to try to reverse the situation tactic, creating disorientation, or otherwise, creating an unexpected situation for the attacker; in order to create; an advantage for the defense (recovery of the ball on the dribble, on the steps or on the shoot). Key words: TRAINING, METHODOLOGY, LEARNING, MOTOR SKILLS, TECHNICAL FUNDAMENTALS.

Vagal intimations for catatonia and electroconvulsive therapy.

Catatonia is currently viewed as a unique syndrome that consists of specific motor signs responding to benzodiazepines and electroconvulsive therapy (ECT). Advances in catatonia may provide a new window into the mechanism of ECT. Findings on catatonia are updated and related to the mechanism of ECT. Selective literature review. There are several putative models and mechanisms of catatonia concerning motor circuitry dysfunction, abnormal neurotransmitters, epilepsy, genetic risk factors, and endocrine and immune dysfunction. Fear and vagal nerve models are presented casting catatonia in an evolutionary-based autonomic neural substrate of social behaviors, engagement, and disengagement according to perceived level of danger. Benzodiazepines and ECT are thought to have autonomic and vagal effects. Advances in catatonia provide a new window into the mechanism of ECT. Fear and vagal nerve models call for further anatomical, functional, and clinical studies on the vagal nerve and for further studies on the use of anticholinergic medications in catatonia, on vagal function in catatonia, and on the effects of benzodiazepines, ECT, and induced seizures on cholinergic and vagal function.

Body side and predominant motor features at the onset of Parkinson's disease are linked to motor and nonmotor progression

Patients with Parkinson's disease most often have asymmetric motor features at onset, and specific motor signs (ie, tremor versus bradykinesia and rigidity) frequently characterize the first few years of disease evolution. Some previous clinical evidence has suggested that body side and a predominance of motor manifestations at disease onset are linked to long-term evolution and disease progression. We prospectively analyzed 206 patients with Parkinson's disease according to the most affected side and predominant motor signs at onset. Patients were divided into left-side rigid-akinetic (n = 71), right-side rigid-akinetic (n = 59), left-side tremor (n = 41), and right-side tremor (n = 35) subgroups. These subgroups were compared in terms of motor and cognitive functions, mean motor deterioration per year (calculated as the motor score divided by disease duration), total equivalent doses of dopaminergic drugs, and the presence of hallucinations and rapid eye movement sleep behavior disorder. Disease duration was similar in all groups. Motor fluctuations were more likely to occur in rigid-akinetic patients. In a multiple model analysis adjusted for potential confounders, faster disease progression was associated with right-side (P = 0.045) and rigid-akinetic onset (P = 0.001). With respect to nonmotor symptoms, the rigid-akinetic type was associated with increased risk of cognitive decline (P = 0.004) compared with the tremor type. A trend was noticed toward an increased risk of developing visual hallucinations in rigid-akinetic patients and toward an increased frequency of rapid eye movement sleep behavior disorder in those who had left-sided onset of symptoms. Our findings corroborate that body side and type of motor signs at the time of diagnosis affect the evolution of motor severity and may also have an impact on some nonmotor manifestations.

Changes in brain glucose metabolism in subthalamic nucleus deep brain stimulation for advanced parkinson's disease

BackgroundDespite its large clinical application, our understanding about the mechanisms of action of deep brain stimulation of the subthalamic nucleus is still limited. Aim of the present study was to explore cortical and subcortical metabolic modulations measured by Positron Emission Tomography associated with improved motor manifestations after deep brain stimulation in Parkinson disease, comparing the ON and OFF conditions. Patients and methodsInvestigations were performed in the stimulator off- and on-conditions in 14 parkinsonian patients and results were compared with a group of matched healthy controls. The results were also used to correlate metabolic changes with the clinical effectiveness of the procedure. ResultsThe comparisons using Statistical parametric mapping revealed a brain metabolic pattern typical of advanced Parkinson disease. The direct comparison in ON vs OFF condition showed mainly an increased metabolism in subthalamic regions, corresponding to the deep brain stimulation site. A positive correlation exists between neurostimulation clinical effectiveness and metabolic differences in ON and OFF state, including the primary sensorimotor, premotor and parietal cortices, anterior cingulate cortex. ConclusionDeep brain stimulation seems to operate modulating the neuronal network rather than merely exciting or inhibiting basal ganglia nuclei. Correlations with Parkinson Disease cardinal features suggest that the improvement of specific motor signs associated with deep brain stimulation might be explained by the functional modulation, not only in the target region, but also in surrounding and remote connecting areas, resulting in clinically beneficial effects.

Catatonia: The Ultimate yet Treatable Motor Reaction to Fear in Autism

Background: Catatonia is a unique syndrome characterized by specific motor signs, at times life-threatening when aggravated by autonomic dysfunction and fever, yet treatable if recognized early. Catatonia occurs in patients with various disorders including autism and related developmental disorders. Sometimes catatonia develops after severe psychological trauma, supporting the view that it is an extreme motor reaction to fear. Methods: Review of literature on the role of fear in the development of catatonia in autism, and its treatment. Results: There are no empirical studies in the literature addressing the role of fear in the development of catatonia in autism. Case-reports support that stressful events often precede the development of catatonia in autistic people and that catatonia in autism is a treatable syndrome, often requiring psychosocial interventions to reduce anxiety, and the use of benzodiazepines, electroconvulsive therapy, and maintenance electroconvulsive therapy for safe reversal. Autistic people may be particularly vulnerable to catatonia due to social, cognitive, and sensory deficits. Conclusion: Further studies are warranted in autistic people, measuring states of anxiety in response to various stressors, and assessing their relationship to catatonia, and applying various treatments including benzodiazepines, electroconvulsive therapy, and psychosocial interventions, in those with catatonia.

Etiopathogenesis of Catatonia

Catatonia has been rediscovered over the last 2 decades as a unique syndrome that consists of specific motor signs with a characteristic and uniform response to benzodiazepines and electroconvulsive therapy. Further inquiry into its developmental, environmental, psychological, and biological underpinnings is warranted. In this review, medical catatonia models of motor circuitry dysfunction, abnormal neurotransmitters, epilepsy, genetic risk factors, endocrine dysfunction, and immune abnormalities are discussed. Developmental, environmental, and psychological risk factors for catatonia are currently unknown. The following hypotheses need to be tested: neuroleptic malignant syndrome is a drug-induced form of malignant catatonia; Prader-Willi syndrome is a clinical GABAergic genetic-endocrine model of catatonia; Kleine-Levin syndrome represents a periodic form of adolescent catatonia; and anti-N-methyl-d-aspartate receptor encephalitis is an autoimmune type of catatonia.

Catatonia is Hidden in Plain Sight Among Different Pediatric Disorders: A Review Article

Over the past two decades, catatonia has been better demarcated in adult psychiatry as a unique syndrome that consists of specific motor signs with a characteristic response to benzodiazepines and electroconvulsive therapy. Pediatric catatonia is considered rare, but may be underdiagnosed, and hence undertreated. Discussed here are the current diagnostic criteria of catatonia in individual cases of children and adolescents diagnosed with childhood disintegrative disorder, Kleine-Levin syndrome, Prader-Willi syndrome, tic disorder, and autoimmune encephalitis, and the effects of benzodiazepines and electroconvulsive therapy. In these cases, catatonia resolved safely once it was recognized and treated properly. Children and adolescents presenting with these disorders should be systematically assessed for catatonia; when the presence of catatonia is confirmed, the use of benzodiazepines and electroconvulsive therapy should be considered. The occurrence of catatonia in such a wide range of child and adolescent disorders supports the view that pediatric catatonia is not so rare, that there are shared elements in the etiology, psychopathology, and pathophysiology of these disorders, and that catatonia is best classified as a unique neurobiologic syndrome.

Cognitive Functions of the Cerebellum

The cerebellum, the often forgotten “little brain” at the back the cerebrum, has gained significant attention in the last two decades (Desmond, 2010). Although traditionally considered limited in function to component processes of motor control, astute clinicians and researchers posited that such a highly organized brain structure composed of approximately as many neurons as in the cerebrum must have some cognitive function or at least exert some influence on cognitive processes. Despite carefully conducted studies yielding convincing data, papers imputing cognitive function to the cerebellum had been banished to the carousel of rejection. As noted in the paper by Henrietta Leiner, “One peer reviewer actually wrote, ‘This couldn’t possibly be true or we would have discovered it long ago.’” Even current neurology handbooks tend to limit discussion on the outcome of cerebellar pathology to specific motor signs and symptoms. A change in the course of tradition has required a strong theoretical basis, creative neuroanatomy, perceptive clinical observation, rigorous neuropsychological testing, all complemented by human brain imaging. Forward-thinking scientists in each of these areas contributed to this special issue of Neuropsychology Review on cognitive functions of the cerebellum. The five papers are written from different perspectives on the cerebellum, its far-reaching yet systematic connections with supratentorial brain structures, its functions, and the challenges faced in presenting discoveries inconsistent with traditional beliefs. Henrietta Leiner, a mathematician, physicist, computer scientist turned neuroscientist, pioneered new horizons in all areas into which she delved. New voices from dogged thinkers formed a critical impetus for rigorous study to challenge cerebellar doctrine about its functions and also its evolutionary purpose. As noted in the current piece, the Leiners (Henrietta and her late husband, Alan) had put forth the proposition that enlargement of the cerebellum in humans, complemented with development of the massive connections with the supratentorium, occurred in step with the emergence of language. Thus, the cerebellum may have enabled rapid language-based thinking and working memory abilities to support speech perception and production. Jeremy Schmahmann relates his scientific pursuit of cerebellar function through anatomy, neurology, and neuropsychology. His story echoes those of other innovators of new concepts about cerebellar functions, indicating resistance from peers in their ability to break set from the indoctrination of the position that the functions of the cerebellum are solely in the motor domain and cannot contribute to higher-order cognition. Dr. Schmahmann’s systematic analysis of the cerebellum’s functions has led him to delineate a set of general principles of its functions. Included is what he has identified as the Cerebellar Cognitive Affective Syndrome, which has been a cornerstone for current theories about autism and other neurological disorders with an affective component. Andreea Bostan and Peter Strick present an anatomically-based argument that structural interactions between cerebellar and supratentorial structures, including the basal ganglia, provide the communication means for the cerebellum to influence higher-order functions. They review their exquisite tracing studies using rabies virus, which enables identification of multi-synaptic connections traversing extensively across brain systems. The success of these studies has been pivotal in providing incontrovertible evidence for a complex system of closed loop bidirectional communication between selective cerebellar and cortical sites and between selective cerebellar and basal ganglial sites in primates. Cherie Marvel and John Desmond provide replicable support from functional imaging studies on the role of selective regions of the cerebellum in verbal working memory performance in humans. Given the role of the cerebellum as contributing to phonological storage (possibly unique to spoken language) and perhaps even extending working memory capacity, these studies are in keeping with the proposition of the Leiners that the cerebellum, with its great size and high-speed connectivity, with selective regions of the supratentorial brain was essential for human language to evolve. Natalie Zahr, Anne-Lise Pitel, Sandra Chanraud, and I present a focused review on how human neuroimaging studies of chronic alcoholism have furthered our understanding of cerebellar function. The review includes studies using a variety of magnetic resonance imaging (MRI) methods, including structural MRI, diffusion tensor imaging (DTI), fMRI, and MR spectroscopy in conjunction with neuropsychological study. The central tenet is that disruption of an executive frontocerebellar network is a major contributor to characteristic behaviors of alcoholics that, on the one hand, sustain alcohol use disorders possibly through cerebellar conditioning mechanisms, and on the other hand, lead to compensation for frontally-based dysfunctions in alcoholism. As imaging methods improve, we may be in the position to visualize and quantify the integrity of structures in vivo currently hidden within the cerebellum, such as the dentate nucleus (cover figure), in addition to assessing their associated functions. Figure 1 Cover image

Signes neurologiques mineurs et troubles envahissants du développement

Many studies have focused on specific motor signs in autism and Asperger's syndrome, but few has been published on the complete range of neurological soft signs (NSS) in children with pervasive developmental disorder (PDD). Scarce are the studies evaluating NSS in children suffering from PDD not otherwise specified (PDDNOS). This study compared performance of 11 autistic children (AD) and 10 children with PDDNOS, with controls matched on age, sex and cognitive performance on Krebs et al.'s NSS scale. Because of the duration of the assessments and specific difficulties encountered in managing some items, an adaptation of the scale had to be made during a pilot study with the agreement of the author. To be eligible, patients had to meet the following inclusion criteria: an age range of 6-16 years, a diagnosis of autistic disorder or PDDNOS based on the DSMIV criteria (American Psychiatric Association 1994). The autism diagnostic interview-revised (ADI-R) was used in order to confirm the diagnosis and to evaluate the association of the symptoms to the severity of the NSS. The childhood autism rating scale (CARS) was completed for the patients in order to evaluate symptoms at the time of the NSS examination. Cognitive ability was assessed with Raven's progressive matrices. Were excluded patients with: history of cerebral palsy, congenital anomaly of the central nervous system, epilepsy, known genetic syndrome, tuberous sclerosis, neurofibromatosis, antecedent of severe head trauma, Asperger's syndrome, obvious physical deformities or sensory deficits that would interfere with neurological assessment, deep mental retardation and recent or chronic substance use or abuse. Healthy controls shared the same exclusion criteria, with no personal history of neurological, psychiatric disorder or substance abuse, no family history of psychiatric disorder and normal or retardation in schooling. All study procedures were approved by the local Ethics Committee (Comité d'éthique, Razi Hospital), according to the declaration of Helsinki. There was no difference between patients and controls with respect to sex, age and cognitive function. All children had an IQ higher than 81. Significant differences were found between AD children and control group in the motor integration function and sensory integration function. Different NSS scores were significantly higher in the PDDNOS group than in controls: the total scores, motor coordination, motor integration function, sensory integration and abnormal movements. Lower performance in motor coordination skills was associated with higher ADI-R communication score in the AD group. No relationship was found between NSS and CARS' total sore. This study confirms the impaired neurological functioning in autistic as well as PDDNOS children. The association of motor impairment with autistic symptoms highlights the argument that motor control problems can be part of the autism spectrum disorders. The lack of relationship between NSS and intellectual aptitude in the clinical sample provides new elements for the neurodevelopment model of the autism spectrum.

Motor Signs Distinguish Children with High Functioning Autism and Asperger’s Syndrome from Controls

While many studies of motor control in autism have focused on specific motor signs, there has been a lack of research examining the complete range of subtle neuromotor signs. This study compared performance on a neurologic examination standardized for children (PANESS, Physical and Neurological Exam for Subtle Signs, Denckla [1974 Developmental Medicine and Child Neurology, 16(6), 729-741]) between a group of 40 boys aged 6-17 with autism and average range IQs and a group of 55 typically developing boys. The Autism group was shown to have significant impairment on several measures of motor control compared to the Control group. Regression analyses revealed that a model including four PANESS variables offered a high level of discrimination in distinguishing boys with high-functioning autism from controls.

Progression of gait disorder and rigidity and risk of death in older persons.

Bradykinesia, gait disturbance, rigidity, and tremor are common motor signs in old age. All of these signs are associated with increased morbidity and mortality, but the extent to which they are progressive is unknown. Study participants were 787 older Catholic clergy members without clinically diagnosed PD, related conditions, or dementia at baseline. They were evaluated annually for up to 7 years, with >95% follow-up participation by survivors. Evaluations included administration of a modified version of the motor portion of the Unified PD Rating Scale (UPDRS), from which previously established measures of the global UPDRS and four specific motor signs were derived. Scores represent the percent of the total possible UPDRS score obtained. At baseline, the global UPDRS score ranged from 0 to 36.3 (mean +/- SD, 7.3 +/- 6.4). It increased by an average of 0.69 unit per year during follow-up, with more rapid progression in older persons, but there was wide variability with no progression in 21% of subjects and annual increases of up to 8.23 units in the remaining 79%. Of 129 persons who died, 106 had follow-up UPDRS data. In a proportional hazards model, risk of death was associated with both the level of the global UPDRS score at baseline and the annual rate of progression (both p < 0.001). Overall, risk of death in subjects who had some worsening of the global UPDRS score was 2.93 times the rate among those without progression (95% CI, 1.32-6.50). Gait disorder/postural reflex impairment and rigidity worsened, but bradykinesia and tremor did not. Risk of death was associated with worsening of gait/posture but not with the other signs. Gait disorder and rigidity, as assessed with the modified UPDRS, are usually progressive in old age. Both the severity of the gait disorder and its rate of progression are strongly associated with risk of death.